Article
CHOP and c-JUN up-regulate the mutant Z α1-antitrypsin, exacerbating its aggregation and liver proteotoxicity.
The Journal of biological chemistry - 18 Sept 2020
Attanasio Sergio, Ferriero Rosa, Gernoux Gwladys, De Cegli Rossella, Carissimo Annamaria, Nusco Edoardo, Campione Severo, Teckman Jeffrey, Mueller Christian, Piccolo Pasquale, Brunetti-Pierri Nicola
Abstract excerpt
α1-Antitrypsin (AAT) encoded by the SERPINA1 gene is an acute-phase protein synthesized in the liver and secreted into the circulation. Its primary role is to protect lung tissue by inhibiting neutrophil elastase. The Z allele of SERPINA1 encodes a mutant AAT, named ATZ, that changes the protein structure and leads to its misfolding and polymerization, which cause endoplasmic reticulum (ER) stress and liver...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.